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Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature
Light chain (AL) amyloidosis is a plasma cell dyscrasia that results in an overproduction of immunoglobulins of the lambda or kappa light chains. These monoclonal ALs begin to form fibrils with each other and exert their toxic effect by depositing in different organs around the body. Disease present...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9653032/ https://www.ncbi.nlm.nih.gov/pubmed/36381809 http://dx.doi.org/10.7759/cureus.30263 |
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author | Bazzi, Talal Kropman, Kory Benjamin, Mark Al-Rammahi, Ali |
author_facet | Bazzi, Talal Kropman, Kory Benjamin, Mark Al-Rammahi, Ali |
author_sort | Bazzi, Talal |
collection | PubMed |
description | Light chain (AL) amyloidosis is a plasma cell dyscrasia that results in an overproduction of immunoglobulins of the lambda or kappa light chains. These monoclonal ALs begin to form fibrils with each other and exert their toxic effect by depositing in different organs around the body. Disease presentation is indistinct, but it is ideal to diagnose this disorder before end-organ damage is caused. Once the diagnosis of AL amyloidosis is confirmed, the best treatment is autologous stem cell transplantation once a candidate is deemed fit for it; however, there are other chemotherapy agents whose patients can be administered until they undergo stem cell transplantation. In this case presentation and systematic review of AL amyloidosis, we discuss a patient who presented with septic shock and further workup leading to a diagnosis of advanced-stage amyloidosis. We also take a deeper look at AL amyloidosis providing a comprehensive review of the disease process and its treatment options. |
format | Online Article Text |
id | pubmed-9653032 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-96530322022-11-14 Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature Bazzi, Talal Kropman, Kory Benjamin, Mark Al-Rammahi, Ali Cureus Internal Medicine Light chain (AL) amyloidosis is a plasma cell dyscrasia that results in an overproduction of immunoglobulins of the lambda or kappa light chains. These monoclonal ALs begin to form fibrils with each other and exert their toxic effect by depositing in different organs around the body. Disease presentation is indistinct, but it is ideal to diagnose this disorder before end-organ damage is caused. Once the diagnosis of AL amyloidosis is confirmed, the best treatment is autologous stem cell transplantation once a candidate is deemed fit for it; however, there are other chemotherapy agents whose patients can be administered until they undergo stem cell transplantation. In this case presentation and systematic review of AL amyloidosis, we discuss a patient who presented with septic shock and further workup leading to a diagnosis of advanced-stage amyloidosis. We also take a deeper look at AL amyloidosis providing a comprehensive review of the disease process and its treatment options. Cureus 2022-10-13 /pmc/articles/PMC9653032/ /pubmed/36381809 http://dx.doi.org/10.7759/cureus.30263 Text en Copyright © 2022, Bazzi et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Internal Medicine Bazzi, Talal Kropman, Kory Benjamin, Mark Al-Rammahi, Ali Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title | Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title_full | Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title_fullStr | Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title_full_unstemmed | Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title_short | Light Chain Amyloidosis Presenting as a Septic Shock: A Case Report and Review of Literature |
title_sort | light chain amyloidosis presenting as a septic shock: a case report and review of literature |
topic | Internal Medicine |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9653032/ https://www.ncbi.nlm.nih.gov/pubmed/36381809 http://dx.doi.org/10.7759/cureus.30263 |
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